Hypopharyngeal liposarcoma, a Rare and Uncommon Entity, a case report

Case Report | DOI: https://doi.org/10.31579/2692-9562/027

Hypopharyngeal liposarcoma, a Rare and Uncommon Entity, a case report

  • Chidiac Frederic 1
  • Brudasca Ioana 1
  • Buiret Guillaume 1*

1Service ORL et chirurgie cervico-faciale, Centre Hospitalier de Valence.

*Corresponding Author: Guillaume Buiret, Service ORL et chirurgie cervico-faciale, Centre hospitalier de Valence, 26000, Valence (France).

Citation: Frederic C, Ioana B, Guillaume B. (2021) Hypopharyngeal liposarcoma, a Rare and Uncommon Entity, a case report. J Clinical Otorhinolaryngology; 3(2); DOI: 10.31579/2692-9562/027

Copyright: © 2021, G Buiret. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

Received: 25 February 2021 | Accepted: 27 April 2021 | Published: 05 May 2021

Keywords: liposarcoma; hypopharynx; dysphagia; rare entity; limbs; MDM2;CDK4

Abstract

Liposarcoma is the most common form of soft-tissue sarcoma, only 3-8% have a head and neck location. They are commonly found in the retroperitoneum and on the limbs, laryngeal and hypopharyngeal presentation are extremely rare and less than 40 cases have been reported in literature. We present the case of a 50-year-old man with no prior history, who developed two contiguous hypopharyngeal liposarcomas.

Introduction

Liposarcoma is the most common form of soft-tissue sarcoma, only 3-8% have a head and neck location. Liposarcomas are malignant tumors of adipose tissue that rarely have a metastatic evolution but can recur locally [1, 2].
They are commonly found in the retroperitoneum and on the limbs, laryngeal and hypopharyngeal presentation are extremely rare [3] and less than 40 cases have been reported in literature [4]. Their evolution is slow and painless with progressive difficulty swallowing, dyspnea and, or dyspnea. They mainly occur in men between with a median of 64 years old[5]. The diagnosis is suspected based on the endoscopic and radiographic aspects of a polypoid, pedunculated mass with adipocytic density. The final diagnosis is histological with FISH amplification or immunochemistry detecting the abnormal amplification of MDM2 and CDK4, which are located on chromosome 12q13-15. The reference treatment is the surgical excision ad integrum with tumor free margins. Adjuvant radiation is controvertes[3].
We report a case of well-differentiated hypopharyngeal liposarcoma, in which a diagnosis of a simple lipoma was initially proposed.

Case report

A 50 years old man presented with chronic dysphagia and regurgitations. He had no prior history of chronic disease, no treatments and no history of tobacco or alcoholic intoxication either. He was in an excellent general condition with no recent loss of weight. He denied throat pain or dyspnea but endorsed frequent oral regurgitation of solids, food blockage and frequent coughing spells.

The nasofibroscopy examination revealed a benign looking ovoid mass measuring 4cm, attached to the posterior pharyngeal wall, overhanging the esophagus mouth, non-hemorrhagic, flexible, with normal mucosa aspect. The cervical examination showed no lymphadenopathies.

The contrast-enhanced neck and chest computed tomography revealed a 2.5cm circumscribed adipose tumor of the hypopharynx, without reaching the larynx and the retro-pharyngeal spaces, no lymph node was associated (Figure 1) 

Figure 1.
Figure 1: Contrast-enhanced CT workup. Axial (A) and coronal (B) views show a well delineated fat-delineated mass arising from the posterior wall of the pharynx

A panendoscopic examination under general anesthesia with biopsies was performed. The anatomopathologic results were in favor of a lipoma. Unfortunately, the patient kept experiencing dysphagia with an aggravation of the regurgitations. 

We decided to perform an endoscopic surgical resection. The surgery consisted of a complete resection of the mass by LASER dissection, performed off of the tumor (Figure 2). A second tumor adjacent to the first one was removed during the same operation.

Figure 2: Endoscopic surgical laser resection of the liposarcoma

Histological examination concluded on two well-differenciated sclerosing liposarcomas, composed of mature adipocytes and CD34+, HMGA2+ spindle cells with positive MDM2 gene amplification. The resection was complete with tumor free margins (Figure 3). 

Figure 3: Nodular but non-encapsulated neoplasm with low to middle cellularity. The cells are spindled with bland nuclear features. The stroma is collagenic, sometimes myxoïd, with lymphocytic infiltrate.  Note the mature adipocytes (arrow).

The one-month and the six months follow-up the patient was asymptomatic. The endoscopies revealed a good healing of the mucosa with no sign of recurrence. The cervical neck palpation was free of lymphadenopathies. The CT-scan at 6 months showed no signs of recurrence either. 

The case was reported to a specialized multidisciplinary reunion, which decided on a biannual clinical and CT surveillance. 

Discussion

Liposarcomas are malignant [1] tumors of adipose tissue that are relatively common in adults, they account as 15-18% of all sarcomas.

The hypopharyngeal localization is extremely rare, with only 28 case reports referenced in Pubmed in 2017, and less than a hundred cases have been reported in literature [6]. 

Clinically, the symptoms are related to the localization adjacent to the pharyngolaryngeal crossroad, with organic dysphagia, sensation of foreign body, dysphonia or dyspnea due to airway obstruction. A nasal endoscopy performed at the consultation shows a sub-mucosal tumor, with respect of the mucosa, which might obliterate the pharyngolaryngeal space [7].

CT or MRI examination reveals one or more well-limited tumor, with fat density. The MRI is more accurate than the CT-scan to delineate tumor boundaries before surgical work-up [4]. The liposarcoma can be easily confused with a benign or malignant tumor of the pharyngolaryngeal space, such as lipoma, fibroma, carcinoma, sarcoma, and lymphoma. The MRI can help to distinguish lipomas from liposarcomas by showing a mosaic heterogeneity in liposarcomas whereas lipomas are preferentially homogenous [1].

The histological staging proposed by Ezinger and Winslow in 1962 [8] is considered as the reference for liposarcomas. Four histological types of liposarcoma are described: well-differentiated, mixoid or round cell, pleomorphic, and dedifferentiated liposarcoma [6]. The most common (40-45%) is the well differentiated, a low-grade lesion with three subtypes: the lipoma-like liposarcoma, the inflammatory and the sclerosing liposarcoma [3]. Liposarcomas do not develop from lipomas but rather from primitive mesenchymal cells1. FISH detection of MDM2 and CDK4 amplification distinguishes welldifferentiated and dedifferenciated liposarcomas from lipomas. If FISH technic is not availabale immunohistochemistry is a reasonable alternative [5]. 

The gold standard treatment is the surgical resection with a cervical or an endoscopic approach, to obtain free margins of resection [3,6]. The second option allows a minimal post-operative morbidity: rapid resumption of feeding, no need for a tracheotomy and decreased length of hospital stay. Systematic neck dissection is not indicated if there are no lymphadenopathies, the rate of nodal metastase is low [7].

The diagnosis established, the case has to be discussed in a specialized multidisciplinary meeting to decide of a potential adjuvant therapy. Adjuvant radiation might be discussed in cases of dedifferenciated liposarcomas or in cases of inoperable tulmors [3, 7].

Metastasis has not been reported and the long-term prognosis is excellent (with an over 90% 5-year survival) [5, 7]. The mortality rate depend on the histological type and location.  However the main risk is the recurrence, it is frequent and can occur at long term [6]. The main risk factor is the incomplete resection [9]. For all these reasons a long term follow up with clinical, CT or endoscopic surveillance is essential. 

Compliance with Ethical Standards

There are no conflicts of interest for the authors in this study. The patient gave an informed consent.

Conclusion

The hypopharyngeal liposarcoma is a rare entity encountered in clinical practice. Despite this, the clinician has to think about this etiology and have recourse to a specialist opinion concerning the surgical management of this tumor and the follow up.

References

Clearly Auctoresonline and particularly Psychology and Mental Health Care Journal is dedicated to improving health care services for individuals and populations. The editorial boards' ability to efficiently recognize and share the global importance of health literacy with a variety of stakeholders. Auctoresonline publishing platform can be used to facilitate of optimal client-based services and should be added to health care professionals' repertoire of evidence-based health care resources.

img

Virginia E. Koenig

Journal of Clinical Cardiology and Cardiovascular Intervention The submission and review process was adequate. However I think that the publication total value should have been enlightened in early fases. Thank you for all.

img

Delcio G Silva Junior

Journal of Women Health Care and Issues By the present mail, I want to say thank to you and tour colleagues for facilitating my published article. Specially thank you for the peer review process, support from the editorial office. I appreciate positively the quality of your journal.

img

Ziemlé Clément Méda

Journal of Clinical Research and Reports I would be very delighted to submit my testimonial regarding the reviewer board and the editorial office. The reviewer board were accurate and helpful regarding any modifications for my manuscript. And the editorial office were very helpful and supportive in contacting and monitoring with any update and offering help. It was my pleasure to contribute with your promising Journal and I am looking forward for more collaboration.

img

Mina Sherif Soliman Georgy

We would like to thank the Journal of Thoracic Disease and Cardiothoracic Surgery because of the services they provided us for our articles. The peer-review process was done in a very excellent time manner, and the opinions of the reviewers helped us to improve our manuscript further. The editorial office had an outstanding correspondence with us and guided us in many ways. During a hard time of the pandemic that is affecting every one of us tremendously, the editorial office helped us make everything easier for publishing scientific work. Hope for a more scientific relationship with your Journal.

img

Layla Shojaie

The peer-review process which consisted high quality queries on the paper. I did answer six reviewers’ questions and comments before the paper was accepted. The support from the editorial office is excellent.

img

Sing-yung Wu

Journal of Neuroscience and Neurological Surgery. I had the experience of publishing a research article recently. The whole process was simple from submission to publication. The reviewers made specific and valuable recommendations and corrections that improved the quality of my publication. I strongly recommend this Journal.

img

Orlando Villarreal

Dr. Katarzyna Byczkowska My testimonial covering: "The peer review process is quick and effective. The support from the editorial office is very professional and friendly. Quality of the Clinical Cardiology and Cardiovascular Interventions is scientific and publishes ground-breaking research on cardiology that is useful for other professionals in the field.

img

Katarzyna Byczkowska

Thank you most sincerely, with regard to the support you have given in relation to the reviewing process and the processing of my article entitled "Large Cell Neuroendocrine Carcinoma of The Prostate Gland: A Review and Update" for publication in your esteemed Journal, Journal of Cancer Research and Cellular Therapeutics". The editorial team has been very supportive.

img

Anthony Kodzo-Grey Venyo

Testimony of Journal of Clinical Otorhinolaryngology: work with your Reviews has been a educational and constructive experience. The editorial office were very helpful and supportive. It was a pleasure to contribute to your Journal.

img

Pedro Marques Gomes

Dr. Bernard Terkimbi Utoo, I am happy to publish my scientific work in Journal of Women Health Care and Issues (JWHCI). The manuscript submission was seamless and peer review process was top notch. I was amazed that 4 reviewers worked on the manuscript which made it a highly technical, standard and excellent quality paper. I appreciate the format and consideration for the APC as well as the speed of publication. It is my pleasure to continue with this scientific relationship with the esteem JWHCI.

img

Bernard Terkimbi Utoo

This is an acknowledgment for peer reviewers, editorial board of Journal of Clinical Research and Reports. They show a lot of consideration for us as publishers for our research article “Evaluation of the different factors associated with side effects of COVID-19 vaccination on medical students, Mutah university, Al-Karak, Jordan”, in a very professional and easy way. This journal is one of outstanding medical journal.

img

Prof Sherif W Mansour

Dear Hao Jiang, to Journal of Nutrition and Food Processing We greatly appreciate the efficient, professional and rapid processing of our paper by your team. If there is anything else we should do, please do not hesitate to let us know. On behalf of my co-authors, we would like to express our great appreciation to editor and reviewers.

img

Hao Jiang

As an author who has recently published in the journal "Brain and Neurological Disorders". I am delighted to provide a testimonial on the peer review process, editorial office support, and the overall quality of the journal. The peer review process at Brain and Neurological Disorders is rigorous and meticulous, ensuring that only high-quality, evidence-based research is published. The reviewers are experts in their fields, and their comments and suggestions were constructive and helped improve the quality of my manuscript. The review process was timely and efficient, with clear communication from the editorial office at each stage. The support from the editorial office was exceptional throughout the entire process. The editorial staff was responsive, professional, and always willing to help. They provided valuable guidance on formatting, structure, and ethical considerations, making the submission process seamless. Moreover, they kept me informed about the status of my manuscript and provided timely updates, which made the process less stressful. The journal Brain and Neurological Disorders is of the highest quality, with a strong focus on publishing cutting-edge research in the field of neurology. The articles published in this journal are well-researched, rigorously peer-reviewed, and written by experts in the field. The journal maintains high standards, ensuring that readers are provided with the most up-to-date and reliable information on brain and neurological disorders. In conclusion, I had a wonderful experience publishing in Brain and Neurological Disorders. The peer review process was thorough, the editorial office provided exceptional support, and the journal's quality is second to none. I would highly recommend this journal to any researcher working in the field of neurology and brain disorders.

img

Dr Shiming Tang

Dear Agrippa Hilda, Journal of Neuroscience and Neurological Surgery, Editorial Coordinator, I trust this message finds you well. I want to extend my appreciation for considering my article for publication in your esteemed journal. I am pleased to provide a testimonial regarding the peer review process and the support received from your editorial office. The peer review process for my paper was carried out in a highly professional and thorough manner. The feedback and comments provided by the authors were constructive and very useful in improving the quality of the manuscript. This rigorous assessment process undoubtedly contributes to the high standards maintained by your journal.

img

Raed Mualem

International Journal of Clinical Case Reports and Reviews. I strongly recommend to consider submitting your work to this high-quality journal. The support and availability of the Editorial staff is outstanding and the review process was both efficient and rigorous.

img

Andreas Filippaios

Thank you very much for publishing my Research Article titled “Comparing Treatment Outcome Of Allergic Rhinitis Patients After Using Fluticasone Nasal Spray And Nasal Douching" in the Journal of Clinical Otorhinolaryngology. As Medical Professionals we are immensely benefited from study of various informative Articles and Papers published in this high quality Journal. I look forward to enriching my knowledge by regular study of the Journal and contribute my future work in the field of ENT through the Journal for use by the medical fraternity. The support from the Editorial office was excellent and very prompt. I also welcome the comments received from the readers of my Research Article.

img

Dr Suramya Dhamija

Dear Erica Kelsey, Editorial Coordinator of Cancer Research and Cellular Therapeutics Our team is very satisfied with the processing of our paper by your journal. That was fast, efficient, rigorous, but without unnecessary complications. We appreciated the very short time between the submission of the paper and its publication on line on your site.

img

Bruno Chauffert

I am very glad to say that the peer review process is very successful and fast and support from the Editorial Office. Therefore, I would like to continue our scientific relationship for a long time. And I especially thank you for your kindly attention towards my article. Have a good day!

img

Baheci Selen

"We recently published an article entitled “Influence of beta-Cyclodextrins upon the Degradation of Carbofuran Derivatives under Alkaline Conditions" in the Journal of “Pesticides and Biofertilizers” to show that the cyclodextrins protect the carbamates increasing their half-life time in the presence of basic conditions This will be very helpful to understand carbofuran behaviour in the analytical, agro-environmental and food areas. We greatly appreciated the interaction with the editor and the editorial team; we were particularly well accompanied during the course of the revision process, since all various steps towards publication were short and without delay".

img

Jesus Simal-Gandara

I would like to express my gratitude towards you process of article review and submission. I found this to be very fair and expedient. Your follow up has been excellent. I have many publications in national and international journal and your process has been one of the best so far. Keep up the great work.

img

Douglas Miyazaki

We are grateful for this opportunity to provide a glowing recommendation to the Journal of Psychiatry and Psychotherapy. We found that the editorial team were very supportive, helpful, kept us abreast of timelines and over all very professional in nature. The peer review process was rigorous, efficient and constructive that really enhanced our article submission. The experience with this journal remains one of our best ever and we look forward to providing future submissions in the near future.

img

Dr Griffith

I am very pleased to serve as EBM of the journal, I hope many years of my experience in stem cells can help the journal from one way or another. As we know, stem cells hold great potential for regenerative medicine, which are mostly used to promote the repair response of diseased, dysfunctional or injured tissue using stem cells or their derivatives. I think Stem Cell Research and Therapeutics International is a great platform to publish and share the understanding towards the biology and translational or clinical application of stem cells.

img

Dr Tong Ming Liu

I would like to give my testimony in the support I have got by the peer review process and to support the editorial office where they were of asset to support young author like me to be encouraged to publish their work in your respected journal and globalize and share knowledge across the globe. I really give my great gratitude to your journal and the peer review including the editorial office.

img

Husain Taha Radhi

I am delighted to publish our manuscript entitled "A Perspective on Cocaine Induced Stroke - Its Mechanisms and Management" in the Journal of Neuroscience and Neurological Surgery. The peer review process, support from the editorial office, and quality of the journal are excellent. The manuscripts published are of high quality and of excellent scientific value. I recommend this journal very much to colleagues.

img

S Munshi

Dr.Tania Muñoz, My experience as researcher and author of a review article in The Journal Clinical Cardiology and Interventions has been very enriching and stimulating. The editorial team is excellent, performs its work with absolute responsibility and delivery. They are proactive, dynamic and receptive to all proposals. Supporting at all times the vast universe of authors who choose them as an option for publication. The team of review specialists, members of the editorial board, are brilliant professionals, with remarkable performance in medical research and scientific methodology. Together they form a frontline team that consolidates the JCCI as a magnificent option for the publication and review of high-level medical articles and broad collective interest. I am honored to be able to share my review article and open to receive all your comments.

img

Tania Munoz

“The peer review process of JPMHC is quick and effective. Authors are benefited by good and professional reviewers with huge experience in the field of psychology and mental health. The support from the editorial office is very professional. People to contact to are friendly and happy to help and assist any query authors might have. Quality of the Journal is scientific and publishes ground-breaking research on mental health that is useful for other professionals in the field”.

img

George Varvatsoulias

Dear editorial department: On behalf of our team, I hereby certify the reliability and superiority of the International Journal of Clinical Case Reports and Reviews in the peer review process, editorial support, and journal quality. Firstly, the peer review process of the International Journal of Clinical Case Reports and Reviews is rigorous, fair, transparent, fast, and of high quality. The editorial department invites experts from relevant fields as anonymous reviewers to review all submitted manuscripts. These experts have rich academic backgrounds and experience, and can accurately evaluate the academic quality, originality, and suitability of manuscripts. The editorial department is committed to ensuring the rigor of the peer review process, while also making every effort to ensure a fast review cycle to meet the needs of authors and the academic community. Secondly, the editorial team of the International Journal of Clinical Case Reports and Reviews is composed of a group of senior scholars and professionals with rich experience and professional knowledge in related fields. The editorial department is committed to assisting authors in improving their manuscripts, ensuring their academic accuracy, clarity, and completeness. Editors actively collaborate with authors, providing useful suggestions and feedback to promote the improvement and development of the manuscript. We believe that the support of the editorial department is one of the key factors in ensuring the quality of the journal. Finally, the International Journal of Clinical Case Reports and Reviews is renowned for its high- quality articles and strict academic standards. The editorial department is committed to publishing innovative and academically valuable research results to promote the development and progress of related fields. The International Journal of Clinical Case Reports and Reviews is reasonably priced and ensures excellent service and quality ratio, allowing authors to obtain high-level academic publishing opportunities in an affordable manner. I hereby solemnly declare that the International Journal of Clinical Case Reports and Reviews has a high level of credibility and superiority in terms of peer review process, editorial support, reasonable fees, and journal quality. Sincerely, Rui Tao.

img

Rui Tao

Clinical Cardiology and Cardiovascular Interventions I testity the covering of the peer review process, support from the editorial office, and quality of the journal.

img

Khurram Arshad